Pyonephrosis is characterized by infective hydronephrosis accompanied by suppurative destruction of the renal parenchyma, resulting in partial or complete impairment of renal function. Patients present with wide range of symptoms, they can be asymptomatic in 15% but most of them present with septic features like fever, chills, and flank pain. Urinary tract infections, functional and anatomic obstruction, and anatomic anomalies such as pelvic and horseshoe kidneys are common etiologic factors. We report a 30 years old female patient with giant pyonephrosis in an ectopic kidney. She presented with acute toxic symptoms after she has been experiencing progressive abdominal swelling and pain for a couple of years. Simple nephrectomy was done four weeks after she has been initially treated with percutaneous drainage of the pus and intravenous antibiotics.